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Pseudomyxoma peritonei is an uncommon tumor known for its production of mucus in the abdominal cavity.[1] If left untreated, mucin will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys, etc.
Unlike most cancers, PMP rarely spreads through the lymphatic system or through the bloodstream. PMP is most commonly associated with cancer of the appendix; mucinous tumors of the ovary have also been implicated.
PMP may be diagnosed with a range of conditions. While the majority of PMP cases are associated with appendiceal carcinomas,[2] other conditions may also be found, including disseminated peritoneal adenomucinosis (DPAM), peritoneal carcinomas, several mucinous cancers (mucinous adenocarcinoma, mucinous cystadenoma, and mucinous cystadenocarcinoma), as well as other disease states.[3] Symptoms may include abdominal or pelvic pain and/or bloating, distension, digestive disorders, weight changes, increased girth and infertility. Diagnosis is confirmed through pathology. Diagnostic tests may include CT scans, and the evaluation of tumor markers.
PMP is commonly discovered during surgery for other conditions, i.e., hernia repair, following which an experienced pathologist can confirm the diagnosis. Due to the rarity of this disease, it is important to obtain an accurate diagnosis so that appropriate treatment may be obtained.
Treatment for PMP is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from watchful waiting to debulking and cytoreductive surgery.[4]
Most commonly, treatment for PMP involves surgery performed by specific specialists trained in treating this disease.
When appropriate, surgery may include intraperitoneal hyperthermic chemotherapy, or post operative systemic chemotherapy.
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